JCI - A hypomorphic mouse model of dystrophic epidermolysis bullosa reveals mechanisms of disease and response to fibroblast therapy

A hypomorphic mouse model of dystrophic epidermolysis bullosa reveals mechanisms of disease and response to fibroblast therapy
J. Clin. Invest. 118:5 doi:10.1172/JCI34292 [Go to this article.]

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Figure 2
Dermal-epidermal separation in the Col7a1^flNeo/flNeo mouse is caused by strongly reduced collagen VII deposition.

(A) Histological analysis (H&E staining) of paw skin of 1-day-old Col7a1^WT/WT and Col7a1^flNeo/flNeo mice shows dermal-epidermal blister formation (asterisk) with erythrocyte accumulation in the latter. (B) Indirect immunofluorescence signal of collagen VII (green) in paw skin of 1-day-old Col7a1^flNeo/flNeo mice was strongly reduced compared with Col7a1^WT/WT mice. Asterisk indicates the blister cavity. Scale bars: 50 μm. (C and D) Ultrastructural analysis showed normal hemidesmosomes (white arrows), but few anchoring fibrils (black arrows), in the skin of Col7a1^flNeo/flNeo mice. Asterisk indicates the blister cavity. Original magnification, ×195,000.